Pulmonary lymphomatoid granulomatosis in a 4 year old

The authors report a pulmonary lymphomatoid granulomatosis (LG) in a 4-year-old girl. The clinicopathologic and radiological features of this rare entity are discussed with special emphasis on differential diagnosis, including a brief literature review. LG is an aggressive multiorgan disease that primarily affects the adult lung. There are no specific presumptive clinical and laboratory findings, including tumor markers and imaging techniques, that distinguish LG from other pulmonary nodular lesions. The most important diagnostic aid is to bear this entity in mind when a child presents with pulmonary nodule associated with intractable long-lasting symptoms. Open lung biopsy and total excision is mandatory for the appropriate diagnosis and treatment.